Chiari Network Associated with Hypoxemia in a Neonate: Case Report and Review of the Literature
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CASE REPORT
Chiari Network Associated with Hypoxemia in a Neonate: Case Report and Review of the Literature Saif Aljemmali1 · John Bokowski1 · Raymond Morales1 · Ra‑id Abdulla2 Received: 12 July 2020 / Accepted: 22 July 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Chiari network is an embryonic remnant of the right sinus venosus. It appears as a thin, fenestrated membrane attached to two or more regions within the right atrium. Usually, a Chiari network has a benign course; however, rare complications associated with a Chiari network have been reported. We present the case of a neonate with hypoxemia and a Chiari network protruding into the right ventricular inflow tract associated with right-to-left shunting across the patent foramen ovale throughout the cardiac cycle. We noted spontaneous improvement after 3 weeks of life. Keywords Chiari network · Eustachian valve · Sinus venosus · Cyanosis · Hypoxemia
Case Report A 5-day-old male was born at 36 weeks of gestation via C-section to a 23-year-old female with no perinatal care. Cardiology was consulted due to failed newborn pulse oximeter screening and a car seat challenge test. The patient was asymptomatic and hemodynamically stable without respiratory distress or cyanosis. However, the patient was hypoxemic, with oxygen saturation 88–90% while on room air. The chest X-ray and electrocardiogram were within normal limits for age. Echocardiography showed normal cardiac anatomy and a patent foramen ovale (PFO) with right-to-left shunting throughout the cardiac cycle. A prominent Chiari network was present. This was attached to the free wall of the right atrium, Eustachian valve, and atrial septum. The Chiari network protruded into the right ventricular inflow tract during diastole without an appreciable pressure gradient across right ventricular through the tricuspid valve. The Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00246-020-02432-w) contains supplementary material, which is available to authorized users. * Saif Aljemmali [email protected] 1
Section of Pediatric Cardiology, Department of Pediatrics, Rush University Medical Center, Chicago, IL, USA
Division of Cardiology, Ann & Robert H. Lurie Children’s Hospital of Chicago; Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL, USA
2
right ventricle, tricuspid, and pulmonary valve sizes were normal. After close monitoring without a clinical change in status, we postulated that there would be a spontaneous improvement of hypoxemia with the natural improvement of right ventricular compliance and pulmonary vascular resistance. The patient was discharged home with a close follow up. Four weeks later, the patient was seen at our clinic, and the oxygen saturation was normal. Repeated echocardiography showed bidirectional shunting, mainly left-to-right across the PFO without the involution of the Chiari network.
Case Discussion and Review of Literature Hans Chiari fir
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